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[Recurrent infantile digital fibromatosis]
1Ortopaedkirurgisk afdeling, Centralsygehuset i Naestved.
Insights
Recurring infantile digital fibromatosis (Reye's tumour) is a rare, non-malignant childhood tumor. Recent literature suggests conservative management due to frequent spontaneous regression, reserving surgery for aggressive cases.
Area of Science:
- Pediatric Oncology
- Dermatopathology
Background:
- Recurring infantile digital fibromatosis (IDF), also known as Reye's tumour, is a rare benign neoplasm affecting infants.
- Typically diagnosed before age three, it presents on the extremities and has an unknown etiology.
Observation:
- Pathognomonic eosinophilic inclusion bodies are observed near the nucleus of tumor cells.
- These inclusions are composed of actin filaments entrapping cellular components.
Findings:
- Approximately 100 cases of recurring infantile digital fibromatosis have been documented.
- The condition is characterized by recurrent tumor formation, primarily in infants.
Implications:
- Current literature advocates for conservative treatment approaches for recurring infantile digital fibromatosis.
- Surgical intervention is recommended only for rare cases with aggressive or uncontrollable fibroma growth, as spontaneous regression is common.
Abstract:
In reflection of a current case, the latest literature concerning recurring infantile digital fibromatosis (Reye's tumour) are summed up. It is a non-malignant, rarely encountered tumour observed in children, appearing on the hands and feet. The tumour is almost exclusively diagnosed before the age of three. The etiology is unknown. Approximately 100 cases have been reported in the literature. Eosinophilic inclusion bodies near the nucleus of the tumourcells are pathognomonic. According to the latest literature, the content of the inclusions is mostly actin filaments between which the formed elements of the cell are entrapped. Previous treatment was repeated excision of the recurring tumours, until spontaneous cessation of disease activity. According to the latest literature, a more conservative treatment is recommended, because the fibromas often regress spontaneously over few years. Therefore surgical treatment is only needed in rare cases with aggressive or uncontrollable growth of the fibromas.